Aplastik Anemia Idiopatik Aplastik Anemia Idiopatik adalah penyakit dimana semua sel darah merah gagal di bentuk

dengan sempurna oleh sumsum tulang. Biasanya penyakit ini disebabkan oleh tindakbalas badan terhadap selnya sendiri (proses autoimunisasi). Gejala Aplastik Anemia Idiopatik Terdapat beberapa gejala yang boleh dikaitkan dengan penyakit Aplastik Anemia Idiopatik ialah:-

Pesakit akan mengalami keletihan yang agak teruk Muka penghidap akan kelihatan agak pucat Akan mengalami sesak nafas jika melakukan aktiviti fizikal Denyutan jantung agak laju dan tidak sekata Mudah mengalami lebam di anggota badan Ruam Hidung bedarah Gusi berdarah Pendarahan yang berpanjangan.

Pemakanan dan rehat seseorang itu boleh mempengaruhi dan boleh mengawal penyakit Anemia Aplastik Idiopatik. Bagi rawatan moden pemindahan darah dan platlet biasanya dilakukan untuk mengatasi masalah ini namun sekiranya penyakit ini berada pada tahap yang serius pemindahan sumsum tulang akan dilakukan. Pemindahan sumsum tulang hanya boleh di lakukan pada mereka yang berusia 40 tahun ke bawah. Ini kerana bagi mereka yang melakukan pemindahan sumsum tulang pada usia ini akan menghadapi risiko tinggi untuk tidak berjaya.

Idiopathic aplastic anemia Idiopathic aplastic anemia is a condition in which the bone marrow fails to properly make blood cells. Bone marrow is the soft, fatty tissue in the center of bones. See also:

Causes

Anemia Secondary (acquired) aplastic anemia

Aplastic anemia results from injury to the blood stem cells, immature cells in the bone marrow that give rise to all of other blood cells types. The injury causes a decrease in the number of every type of blood cell in the body -- red cells, white cells, and platelets. Low numbers of red cells, white cells, and platelets is a condition called pancytopenia. Idiopathic means the cause is unknown. However, idiopathic aplastic anemia is thought to occur when the body reacts against its own cells. This is called an autoimmune disorder. The disease may be acute or chronic, and may get worse over time. There are no known risk factors. Aplastic anemia may also be caused by certain medical conditions (such as pregnancy or lupus) or exposure to some toxins or drugs (including chemotherapy). See: Secondary (acquired) aplastic anemia In some cases, aplastic anemia is associated with another blood disorder called paroxysmal nocturnal hemoglobinuria (PNH).

Symptoms
Symptoms are the result of bone marrow failure and the loss of blood cell production. Low red cell count (anemia) leads:

Fatigue Pallor (paleness) Rapid heart rate Shortness of breath with exercise Weakness

Low white cell count (leukopenia) causes an increased risk of infection. Low platelet count (thrombocytopenia) results in bleeding, especially of the mucous membranes and skin. Symptoms include:

Bleeding gums Easy bruising Frequent or severe infections Nose bleeds

Exams and Tests

Low red blood cell count (anemia) Low white blood cell count Low reticulocyte count (reticulocytes are immature red blood cell) Low platelet count Bone marrow biopsy shows an overall decrease in the number of blood cells

Treatment
Mild cases of aplastic anemia may be treated with supportive care or may require no treatment. In moderate cases, blood transfusions and platelet transfusions will help correct the anemia and the risk of bleeding. However, over time, blood transfusions may stop working. Too much iron may build-up in the body tissues may occur because of the many transfusions. See Hemochromatosis Severe aplastic anemia occurs when blood-cell counts are very low, and is a life-threatening condition. Bone marrow transplant or stem cell transplant is recommended for severe disease in younger patients. However, they must have a donor who is a sibling (brother or sister) and a good match. This is called a matched sibling donor. For older patients, or for those who do not have a matched, related bone marrow donor, anti-thymocyte globulin or other drugs that suppress the immune system may allow the bone marrow to once again make blood cells. A bone marrow transplant with an unrelated donor may be tried if these medications do not help, or if the disease comes back after getting better.

Outlook (Prognosis)
Untreated aplastic anemia leads to rapid death. Bone marrow transplant has been successful in young people, with long term survival rates of about 80%. Older people have a survival rate of 40 - 70%.

Possible Complications

Severe infections or bleeding Complications of bone marrow transplant (graft failure or graft-versus-host disease) Reactions to medications (patients can have reactions to anti-thymocyte globulin)

When to Contact a Medical Professional

Call your health care provider or go to the emergency room if bleeding occurs for no reason, or if bleeding is difficult to stop. Call if you notice frequent infections or unusual fatigue.

Prevention
There is no known prevention for idiopathic aplastic anemia.

Alternative Names
Anemia - idiopathic aplastic

References
Castro-Malaspina H, O'Reilly R. Aplastic anemia and related disorders. In: Goldman L, Ausiello D, eds. Cecil

Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 171. Update Date: 3/2/2010
Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.